This project is designed to operate a 6-bed Clinical Research Center for Children with facilities for multi-disciplinary clinical investigations. Emphasis will be on combined clinical and basic research. Heritable disorders in childhood will be investigated, with special attention to defining the basic biochemical defects and their genetic implications, through tissue culture studies, electronmicroscopy and other histochemical determinations. Metabolic and endocrine disorders shall be investigated using refined biochemical technics, with the objective of developing new diagnostic and therapeutic tools. Infants and children with cardiopulmonary problems will be studied by quantitative angiocardiography and high-fidelity pressure recordings for myocardial performance evaluation. New diagnostic procedures will be utilized to evaluate cardiac function before and after cardiopulmonary bypass or profound hypothermia. Other projects are aimed at improving our knowledge of certain connective tissue, hematologic, renal and neuromuscular disorders in childhood. A comprehensive study of muscular dystrophy in children, including its treatment, will continue. The efficacy of chemotherapy and of x-irradiation for the treatment of childhood malignancies will be investigated. The biochemical and hormonal correlates of hypertension in children will also be investigated. Some immunologic aspects of organ transplantation will be studied.